| Budget Amount *help |
¥5,200,000 (Direct Cost: ¥4,000,000、Indirect Cost: ¥1,200,000)
Fiscal Year 2014: ¥1,040,000 (Direct Cost: ¥800,000、Indirect Cost: ¥240,000)
Fiscal Year 2013: ¥2,080,000 (Direct Cost: ¥1,600,000、Indirect Cost: ¥480,000)
Fiscal Year 2012: ¥2,080,000 (Direct Cost: ¥1,600,000、Indirect Cost: ¥480,000)
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| Outline of Final Research Achievements |
We performed neuropathological examinations on two autopsied brains from patients with spinocerebellar ataxia type 31 (SCA31). We found there were two degenerating pathways of Purkinje cells with or without halo-like structures. In Purkinje cells with halo-like structures, the nuclear deformity and fragmentation of the Golgi apparatus were more frequently observed than in those without halo-like structures.
Using biotin-labeled (UGGAA)n probe, we tried to identify proteins that bound to pre-messenger RNA with abnormally expanded (UGGAA) repeat in SCA31 brains. As a result, a protein identified in our experiments was a mitochondrial inner membrane protein. This might be false positive, but based on neuropathological observations, it is possible that abnormal pre-messenger RNA could pass through fragile nuclear membrane and interact with proteins involving cytoplasmic organelles in SCA31.
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